ICD-11 classes
08 Diseases of the nervous system
Human prion diseases
8E02 — Genetic prion diseases
8E02.1 — Gerstmann-Straussler-Scheinker syndrome
ICD-11 8E02.1 — Gerstmann-Straussler-Scheinker syndrome
A disease caused by inheritance of mutation(s) in normal prion protein genes. This disease is characterised by cerebellar ataxia, decreased coordination, dysmetria, or dysarthria, and is fatal. Confirmation is by pathological examination of the brain and genetic testing.
The diagnosis includes nothing.
It excludes 1 item.
- Gerstmann syndrome (MB4C)
It has no clarifying diagnoses.
The diagnosis is coded elsewhere:
- Genetic prion diseases #1441